Journal of Japan Society of Perinatal and Neonatal Medicine
Online ISSN : 2435-4996
Print ISSN : 1348-964X
Volume 60, Issue 2
Journal of Japan Society of Perinatal and Neonatal Medicin
Displaying 1-25 of 25 articles from this issue
Review
  • Katsumi Mizuno
    2024Volume 60Issue 2 Pages 187-196
    Published: 2024
    Released on J-STAGE: September 06, 2024
    JOURNAL FREE ACCESS

     For preterm infants, especially very low birth weight infants(VLBWI)and high risk infants with GI tract diseases and congenital heart diseases, the first choice of enteral nutrition is mother’s own milk(MOM)to prevent complications such as necrotizing enterocolitis. Other mothers’ unpasteurized milk has been given to preterm infants if MOM is not available, however, due to the contagious issues, unpasteurized other mothers’ milk is not safe for very preterm infants.

     In 2019, Japan Pediatric Society issued the policy statement of enteral nutrition for preterm and very low birth weight infants”, establishing a system to recommend donor human milk as in developed countries. As of June 2023, more than 80 NICUs have DHM program, however, considering the number of VLBWIs, human milk banking system is not being established yet. Further accumulation of evidence regarding the efficacy and safety of donor human milk is mandatory.

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Originals
  • Tomohiro Sunouchi, Shohei Takami, Kazuko Obana, Atsushi Nakao, Ryu Yon ...
    2024Volume 60Issue 2 Pages 197-204
    Published: 2024
    Released on J-STAGE: September 06, 2024
    JOURNAL FREE ACCESS

     Evidence on the content and duration of perioperative prophylactic antibiotics in neonatal surgery in Japan is scarce. In this study, we focused on the use of perioperative prophylactic antibiotics for children admitted to our NICU/GCU, a general hospital with a perinatal center, and collected and analyzed data to clarify the current situation. The subjects were neonates who underwent surgery in our pediatric surgery center or were hospitalized in the NICU/GCU during the perioperative period. We conducted a retrospective study based on medical records. The study period was 10 years, from January 2013 to December 2022, and 169 cases were analyzed. 15 cases of Surgical Site Infections(SSIs)occurred, with an incidence rate of 8.9%. Perioperative prophylactic antibiotics agents of different spectrums were often chosen in comparison to adult and international neonate guidelines. Although the duration of administration tended to be longer in many procedures, comparison of SSI incidence rates by the number of days of administration suggested that long-term administration may not contribute to SSI prevention.

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  • Yuji Orita, Takashi Hashimoto, Takatsugu Maeda, Yoshiki Naito, Tomonor ...
    2024Volume 60Issue 2 Pages 205-212
    Published: 2024
    Released on J-STAGE: September 06, 2024
    JOURNAL FREE ACCESS

     Objective: To evaluate the changes in the behaviors of obstetrical care providers and maternal outcomes transferred to Kagoshima City Hospital or Kagoshima University Hospital with postpartum hemorrhage before and after the introduction of a simulation-based training and standardized protocol.

     Materials and methods: Patients transferred to Kagoshima City Hospital or Kagoshima University Hospital were included in this study. Data collected before(period 1: 2015‒2017)and after(period 2: 2018‒2020)program initiation were compared. There were 63 and 105 patients referred during periods 1 and 2. Data from medical records were used to compare the behaviors of providers and patient outcomes. Relationships between massive transfusion and other variables were analyzed using logistic regression analysis.

     Results: The rate of shock index recording increased from 11.1% to 39.0% and intravenous line usage ≥20 gauge increased from 92.1% to 100%. In addition, massive transfusion rate significantly decreased from 46.0% to 29.5%(p<0.05). The clinical factors related to massive transfusion were program attendance, cesarean section, blood loss ≥ 2,200g, and coagulopathy.

     Conclusions: The introduction of simulation-based training and a standardized protocol may contribute to earlier awareness of maternal crises and earlier decision to transfer which changed the behaviors of providers and decreased the rate of massive transfusion for postpartum hemorrhage.

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  • Fuka Matsui, Masakatsu Sase, Yuki Nishimoto, Ichiro Miwa, Hiromi Sanai ...
    2024Volume 60Issue 2 Pages 213-219
    Published: 2024
    Released on J-STAGE: September 06, 2024
    JOURNAL FREE ACCESS

     Objective: To investigate changes in the management of pregnancies complicated by epilepsy and their perinatal outcomes. Methods: A retrospective investigation of 104 pregnancies complicated by epilepsy performed between January 2007 and December 2022 at our institution. Results: Complications included seven miscarriages, one stillbirth, and 96 live births. The proportion of pregnancies complicated by epilepsy gradually increased, reaching 1.25% in 2022. Antiepileptic seizure medications were being used in 64.4% of cases at the time of pregnancy diagnosis, with the use of less teratogenic drugs increasing in recent years. The use of concomitant folic acid prior to pregnancy has increased, with 80-100% of pregnant women taking it in the last four years. One fetal death was recorded, while 96 infants survived. There were 3 cases of neonatal anomalies, which is similar to controls. Ten infants delivered from the mothers using antiepileptic seizure medications at delivery required management for drug withdrawal syndrome. Conclusion: Appropriate antiepileptic drug selection and folic acid should be used before conception, the pregnancy and postpartum period should be closely managed, and the newborn delivered in a facility with appropriate care.

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  • Megumi Shibata, Rina Akaishi, Koushi Yamaguchi, Shinji Tanigaki, Nobua ...
    2024Volume 60Issue 2 Pages 220-224
    Published: 2024
    Released on J-STAGE: September 06, 2024
    JOURNAL FREE ACCESS

     There is a combination of age groups with low rubella antibody titers due to the complex rubella vaccination schedule prior to 1995. These complicated titers make serologic interpretation difficult. We analyzed fluctuations in the hemagglutinin inhibition of the rubella virus(RV-HI)and RV-IgM titers in pregnant women between January 2015 and August 2021. We then investigated the interpretation of serology and perinatal outcomes. Rubella infection could not be excluded in 345 patients according to serological testing, symptoms, or medical history. The 345 patients were classified into 4 groups: Group I, rubella HI ratio <4×, IgM antibody-negative; Group II, rubella HI ratio <4×, IgM antibody-positive; Group III, rubella HI ratio 4×, IgM antibody-negative; and Group IV, rubella HI ratio 4×, IgM antibody-positive or positive conversion. Group I included 297 patients (86.1%)without rubella infection. In Group II, 43 patients(12.5%)were diagnosed with persistent IgM. Among Groups II to IV, the possibility of infection could not be ruled out in seven cases, four of which required invasive tests. Three patients were polymerase chain reaction(PCR)-negative and were determined to have a high antibody titer due to the booster effect, while one patient was PCR-positive and was diagnosed with congenital rubella syndrome.

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  • Hiromu OKAKI, Haruka MUTO, Yumi HISABAYASHI, Yoshihiro KURITANI, Airi ...
    2024Volume 60Issue 2 Pages 225-228
    Published: 2024
    Released on J-STAGE: September 06, 2024
    JOURNAL FREE ACCESS

     [Objective]This study aimed to evaluate the risk factors for manual placenta removal and their contribution to the risk of its recurrence.

     [Methods]This retrospective cohort study included women with two consecutive singleton vaginal deliveries who had their latest delivery within a 6-year period from 2017 at our hospital. The primary outcome was the incidence of manual placental removal. Associations between the outcomes and previous manual placenta removal, maternal age, assisted reproductive technology(ART)pregnancy, and past artificial abortion were evaluated.

     [Results]Based on the inclusion and exclusion criteria, 2, 488 women were analyzed, of which 30 required manual placenta removal(1.21%). The univariate analysis revealed significant differences in the following risk factors for manual placenta removal: 1)previous manual placenta removal, 2)ART pregnancy, and 3)age at delivery ≥ 35 years. In the multivariate analysis, previous manual placenta removal(adjusted odds ratio[aOR], 14.71; 95% confidence interval[95%CI], 2.69‒61.57)and ART pregnancy(aOR 8.19, 95%CI 3.20‒19.49)were identified as independent risk factors associated with the risk of manual placenta removal.

     [Conclusions]In cases of past manual placental removal or ART pregnancy, careful management is recommended due to the higher risk of manual placenta removal”.

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  • Shino KUBOTA, Masuyo MATSUMOTO, Megumi KANEKO, Misato OGINO, Shinya TA ...
    2024Volume 60Issue 2 Pages 229-233
    Published: 2024
    Released on J-STAGE: September 06, 2024
    JOURNAL FREE ACCESS

     Mycoplasma Hominis(MH)is one of the normal genital flora in the urogenital tract of men and women. Whether MH is pathogenic or not remain to be clarified. The aim of this study was to investigate the prevalence of MH in pregnant women and association of MH with pregnancy outcomes. In this study, we investigated 616 specimens from 602 pregnancies. 21 specimens(3.4%)were positive for MH. Regarding maternal background, in the MH(+)group, the median age was significantly lower and Bacterial Vaginosis(BV)score ≧ 4 was significantly higher(p value: < 0.01, < 0.01). Of the 454 pregnancies for which the outcome was known, there were significantly more cases of threatened preterm labor in the MH(+)group(p value: < 0.01). However, there was no statistically significant association between the presence of MH and mean delivery weeks, preterm labor, preterm premature rupture of the membranes and chorioamnionitis(p value: 0.226, 0.082, 0.862, 0.756). In this study, BV score ≧ 4 were significantly more common in the MH(+)group. Previous study showed that there was a relationship between MH and bacterial vaginosis. Further research is needed on the possibility of mixed MH and other bacterial infections affecting perinatal outcomes.

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  • Kohei Nomura, Hiroshi Suzumura, Manabu Miyamoto, Masaya Kato, Yoshiyuk ...
    2024Volume 60Issue 2 Pages 234-239
    Published: 2024
    Released on J-STAGE: September 06, 2024
    JOURNAL FREE ACCESS

     The standard first dose of ibuprofen for patent ductus arteriosus of preterm infants is 10mg/kg. However, there has been no report investigating the effect and side effects if ibuprofen first dose was ≦ 5mg/kg.

     We compared the urine output of pre- and post-first ibuprofen therapy, serum creatinine level, and therapy effect up to three doses between the standard(first dose 10mg/kg)and low(first dose 3‒5mg/kg)dose groups. Consequently, the urine output of post-ibuprofen therapy significantly decreased in the standard dose group and had no significant change in the low dose group. Serum creatinine levels mildly elevated in both groups. Efficacy rate up to three doses was not different between both groups(66.7% vs 81.5%).

     As low-dose ibuprofen therapy does not decrease urine output and has equivalent effect compared with the standard therapy, it is a valuable treatment method for patent ductus arteriosus.

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Case reports
  • Hiroaki Wakamatsu, Masayuki Kanno, Masaki Shimizu
    2024Volume 60Issue 2 Pages 240-244
    Published: 2024
    Released on J-STAGE: September 06, 2024
    JOURNAL FREE ACCESS

     Amniotic band syndrome is a syndrome including multiple malformations of the extremities, cranium, face, and trunk caused by the amniotic band that is formed when the amnion ruptures in the fetal period. The type of malformations depend on the timing of amniotic rupture. In case of early rapture, the symptoms become more severe: such as cranial, facial, and thoracoabdominal wall defects. In the present case, a meningocele and scoliosis were noted at fetal period. After birth, we found extensive body wall defects adjacent to the meningocele, and the liver, spleen, and lungs were visible through the peritoneal-like membrane. Considering the risk of injury and infection of the exposed organs, the body wall defect was covered using an urgent flap surgery and skin grafting. The constriction ring of toe and clubfoot were found, therefore we diagnosed the body wall defect as a symptom of amniotic band syndrome. In this case, there was also a finding suspected of chondrodysplasia punctata brachytelephalangic type, and although the contribution to amniotic band syndrome was not clear, the influence of maternal anti-TNF-alpha antibody preparations was speculated. Amniotic band syndrome should be considered in cases with various malformations of different developmental stages and with characteristic findings such as constriction rings.

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  • Hiroaki Makino, Akio Kamiya, Aya Yoshida, Mamoru Morikawa, Hidetaka Ok ...
    2024Volume 60Issue 2 Pages 245-249
    Published: 2024
    Released on J-STAGE: September 06, 2024
    JOURNAL FREE ACCESS

     Pregnancy-related ovarian endometrioma poses a high risk of complications, such as ruptures, torsions, and peripartum complications(e.g., preterm birth, cesarean section). Particularly, large cysts located in the rectouterine pouch often necessitate cesarean section due to interference with fetal descent. Moreover, reports on peripartum cyst reduction procedures to avoid cesarean section are limited. In this report, we present four patients with large rectouterine pouch during pregnancy for whom we proposed transvaginal ultrasound-guided aspiration. One patient did not agree to the procedure. For the remaining patients, aspiration timing varied based on fetal maturity and potential for cyst regrowth(14-16 weeks in two cases and 35 weeks in one case). All procedures concluded without complications, with an average cyst diameter reduction from 6.1 to 3.1 cm. Similarly, all three cases had successful vaginal deliveries with favorable perinatal outcomes. This report demonstrates the potential of transvaginal ultrasound-guided aspiration as a safe and minimally invasive strategy for reducing rectovaginal endometrial cysts, effectively avoiding cesarean section.

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  • Kana Hayashi, Yuichiro Kato, Noriko Takatsuji, Shiori Tannaka, Kenji A ...
    2024Volume 60Issue 2 Pages 250-255
    Published: 2024
    Released on J-STAGE: September 06, 2024
    JOURNAL FREE ACCESS

     A 30-year-old primigravid, nulliparous woman was diagnosed with uterine fibroid 6 cm in diameter, and had been followed up. She became pregnant by embryo transplantation and was referred to our hospital at the 7th week of gestation. She was admitted at 13 weeks and 3 days of pregnancy due to hyperemesis, and an abnormally high blood Ca level of 16.0 mg/dL. Because of a low intact-parathyroid hormone(PTH)and high parathyroid hormone-related peptide(PTHrP)and 1, 25-dihydroxyvitamin D levels, a PTHrP-producing tumor was suspected. We did not find malignant PTHrP-producing tumor. Although the blood Ca level decreased by fluid replacement, it increased again with mild impairment of renal function. As the uterine fibroid was suspected to be a PTHrP-producing tumor, abdominal myomectomy was performed at 15 weeks and 5 days of pregnancy. Both the Ca and PTHrP levels decreased on day 1 after surgery with relief of symptoms, and she was discharged. Histopathological examination indicated uterine leiomyoma accompanied by liquefaction degeneration, and the uterine fibroid was diagnosed by immunostaining as a PTHrP-producing tumor.

     Since uterine fibroid producing PTHrP tumor is rare, the case is reported with a review of the literature.

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  • Ayaka Konta, Norikazu Watanabe, Natsumi Abe, Ai Sato, Nanako Nakai, Ke ...
    2024Volume 60Issue 2 Pages 256-260
    Published: 2024
    Released on J-STAGE: September 06, 2024
    JOURNAL FREE ACCESS

     We report a case of a patient who was referred for hepatic dysfunction during pregnancy and diagnosed with maternal intestinal obstruction due to malrotation of intestine. The patient was 27 years old, in her first pregnancy, who conceived spontaneously, and her previous physician managed her pregnancy. She had nausea and anorexia from the 30th week of pregnancy and lost 5 kg of weight from the 31st to 33rd week of pregnancy. The gastroenterologist pointed out liver dysfunction and referred her to our hospital at 33 weeks and 5 days gestation. Maternal ultrasound showed dilatation of the upper gastrointestinal tract, so a contrast-enhanced CT was performed. CT showed no abnormality in the liver, but intestinal malrotation, torsion, and obstruction were indicated. No intestinal strangulation was observed, and her symptoms and liver dysfunction slowly improved with intravenous nutrition and a nasogastric tube. However, her distress increased, and a cesarean section was performed at 35 weeks and 3 days of pregnancy. Intraoperative observation revealed intestinal obstruction due to malrotation, but it was resolved spontaneously. The patient resumed oral intake and was discharged on postoperative day 10. The patient was considered to have developed liver dysfunction due to persistent malnutrition, and it was the result of intestinal obstruction, with the background of malrotation, due to compression against the pregnant uterus. If liver dysfunction occurs during pregnancy and the upper gastrointestinal tract is dilated, as in this case, intestinal obstruction is a differential.

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  • Eriko Ueda, Sanae Ide, Kensuke Suzuki, Tao Fujioka, Syusuke Amagata, A ...
    2024Volume 60Issue 2 Pages 261-265
    Published: 2024
    Released on J-STAGE: September 06, 2024
    JOURNAL FREE ACCESS

     Circular shunt(CS)is considered to be the most severe form of Ebstein’s disease. Severe Ebstein’s disease has a high risk of fetal hydrops and intrauterine fetal death, and as a result, premature delivery is inevitable. In recent years, the efficacy of maternal Non-steroidal anti-inflammatory drugs(NSAIDs)administration for the most severe Ebstein’s disease has been reported.

     Case: 29 years old, G3P2. At 23 weeks, tricuspid regurgitation(TR), pulmonary valve stenosis, and right atrial dilatation were pointed out. In our hospital, She was diagnosed with Ebsteinʼs disease with CS by fetal echocardiography. Cardiac enlargement progressed and a small amount of ascites developed at 32+1 weeks, and she was hospitalized. maternal administration of Non-steroidal anti-inflammatory drugs(NSAIDs)was started at 32+3 weeks. Due to decreased amniotic fluid due to a side effect, administration was interrupted and the dose was reduced as appropriate. There was no significant improvement in cardiac enlargement, but there was no progression to hydrops fetalis and weight gain was also observed. After consultation with related departments, he was born by planned caesarean section at 37+3 weeks(2,963 g, Apgar score: 8(1 min)/8(5 min)). Postnatal ultrasonography showed no discrepancy with fetal diagnosis, and a modified Starnes operation was performed at 15 days, and the patient was discharged at 89 days. Glenn operation were performed at 8 months of age. The patient is now 1 year and 3 months old, and the postoperative course is good.

     Maternal administration of NSAIDs may suppress the progression of heart failure in severe Ebstein’s disease, prolong the gestation period, and improve the prognosis.

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  • Tomoko TAGUCHI, Asami ITO, Kenichi TAKENOKO, Minako YOKOYAMA, Maika OI ...
    2024Volume 60Issue 2 Pages 266-270
    Published: 2024
    Released on J-STAGE: September 06, 2024
    JOURNAL FREE ACCESS

     In Aomori Prefecture, due to its geographical features and climatic conditions, there are delivery facilities that require time for transport to a perinatal center, and appropriate measures are especially required for patients with critical obstetric hemorrhage.

     We experienced a case of critical obstetric hemorrhage that could have been saved by appropriate cooperation between the hospital and the perinatal center. After forceps delivery at Clinic A, a patient with a vaginal wall laceration lost more than 2, 000 mL of blood, with resulting in a Shock index(SI)exceeding 2.0.

     We judged that the patient’s life was likely to be endangered considering the time required for transport to our hospital. so we first ordered transport after emergency treatment to Hospital B, about 15 minutes from A. At B, pressure hemostasis using rolled gauze and blood transfusion were performed, and the patient was transported to our hospital after her condition was stabilized. When the gauze was removed after arrival, there was a large amount of arterial bleeding from the vaginal wall, and the SI > 2.0 was observed again. The patient was successfully treated with arterial embolization while receiving a pumping transfusion to stop the bleeding.

     We were able to save her life through appropriate collaboration between Clinic A, Hospital B, and our hospital. In a perinatal environment such as our prefecture, hospital collaboration should be considered to save the life of the mother.

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  • Tomo Yamagata, Maya Komatsu, Yoshitsugu Chigusa, Yosuke Kawamura, Suna ...
    2024Volume 60Issue 2 Pages 271-275
    Published: 2024
    Released on J-STAGE: September 06, 2024
    JOURNAL FREE ACCESS

     Neonatal alloimmune thrombocytopenia(NAIT)is a rare but severe pregnancy-associated disease caused by human platelet antigen(HPA)incompatibility between the mother and fetus. The principal complication of NAIT is intracranial hemorrhage(ICH), which poses a substantial risk of perinatal mortality. Although the platelet counts of neonate tends to be lower during subsequent pregnancies following its manifestation during pregnancy with an older sibling, effective preventive perinatal management has not been established. In this report, we present the case of a 38-year-old woman, gravida 4 para 2. Her second child exhibited systemic petechial hemorrhage immediately after birth, accompanied by a platelet count of 30×103/μL. Anti-HPA-4b antibodies were detected in the mother, and NAIT was suspected. During the subsequent conception with her third child, amniotic fluid analysis revealed the presence of HPA-4a/b in the fetus. Since there was concern that the baby might develop severe NAIT, intravenous immunoglobulin was administered every week starting at 30 weeks gestation, and the patient delivered the baby vaginally at 39 weeks gestation. The infant’s platelet count was 60×103/μL, exhibiting spontaneous recovery with an absence of significant complications. When NAIT is suspected during pregnancy with an older sibling, HPA typing via amniocentesis and intravenous immunoglobulin therapy could emerge as promising strategies for enhancing the perinatal prognosis in subsequent pregnancies.

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  • Ryoichi Sakuma, Shin Fujiwara, Rie Fukuhara, Ryo Furukawa, Sayaka Fuji ...
    2024Volume 60Issue 2 Pages 276-282
    Published: 2024
    Released on J-STAGE: September 06, 2024
    JOURNAL FREE ACCESS

     We encountered three cases of giant sacrococcygeal teratoma diagnosed prenatally. One patient experienced cardiac arrest during tumor resection, presumably due to blood loss and hyperkalemia, and required cardiopulmonary resuscitation. In cases of giant sacrococcygeal teratoma, the mortality rate in neonates who undergo surgery within 24 hours after birth is as high as 24%, and careful management is required to prevent bleeding. Because rapid intravenous blood transfusion is required in the event of massive bleeding, securing an intravenous line with an appropriately large diameter is safer. Based on the experience of case 1, our hospital has made it our first choice to secure two 24G, and if possible, 22G peripheral venous lines in the upper limbs; if this is difficult, an external jugular vein cutdown method by a pediatric surgeon will be used. In cases 2 and 3, we were able to administer rapid intravenous blood transfusions. In addition, to adjust the appropriate transfusion dose without excess or deficiency, it is desirable to monitor circulatory control, other than intraoperative blood pressure and heart rate.

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  • Mana Ueda, Sayako Hamazaki, Makoto Oshiro, Atsushi Nakayama, Atsuko Te ...
    2024Volume 60Issue 2 Pages 283-286
    Published: 2024
    Released on J-STAGE: September 06, 2024
    JOURNAL FREE ACCESS

     We report a case of mother-to-child transmission of Group B Streptococcus (GBS). The mother was observed to have GBS in her vaginal culture at 36 weeks of gestation. At 40 weeks and 6 days, she was diagnosed with clinical chorioamnionitis and underwent an emergency cesarean section 4 hours later. Blood culture confirmed GBS bacteremia, and she received 14 days of antimicrobial therapy before being discharged without any complications. The infant had breathing difficulties and required manual ventilation via a facemask. The infant was admitted to the neonatal intensive care unit and received oxygen therapy. During the admission of the infant, it was discovered that the infant had GBS in their blood culture, leading to the diagnosis of early-onset GBS bacteremia. Fortunately, the respiratory distress improved quickly after 10 days of antimicrobial therapy, and the infant was discharged without further complications. Although there have been very few reported cases of the serotype IV of GBS being present in the mother and infant, it is believed to be highly invasive. Therefore, we anticipate that it will be identified as a target serotype for future GBS vaccines.

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  • Yukako Ono, Junko Yoshimoto, Mari Akiyama, Yousuke Washio, Tomoka Okam ...
    2024Volume 60Issue 2 Pages 287-291
    Published: 2024
    Released on J-STAGE: September 06, 2024
    JOURNAL FREE ACCESS

     We report a case of partial trisomy of the long arm of chromosome 13 with holoprosencephaly(HPE)in a female child. She had a microcephaly, hypotelorism, nasal septal defect, right cleft lip and palate and polydactyly. A brain magnetic resonance image revealed the lobar HPE. She developed West syndrome at the age of three months and died aged four years and two months. The chromosome karyotype was 46,XX,rec(13)dup(13q)inv(13)(p12q14.1)mat, derived from her mother’s inter-arm inversion. The 13q partial trisomy phenotype is milder than that of 13 trisomy and is reported to have a better prognosis. According to previous reports, HPE is not a common phenotype of partial trisomy 13q. More cases need to be studied to clarify the relationship between the partial trisomy 13q phenotype and duplication site.

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  • Ayano FUJII, Tokumasa SUEMITSU, Takahiro MITANI, Mizuho KADOOKA, Akira ...
    2024Volume 60Issue 2 Pages 292-297
    Published: 2024
    Released on J-STAGE: September 06, 2024
    JOURNAL FREE ACCESS

     Arrhythmias during pregnancy tend to worsen because of increased circulating blood volume and changes in the autonomic function; hence, pre-pregnancy treatment interventions are essential.

     Non-sustained ventricular tachycardia without any underlying disease has a good prognosis and can be managed with pharmacological therapy. However, sustained ventricular tachycardia can lead to hemodynamic failure and ventricular fibrillation, which are life-threatening and require immediate therapeutic intervention. Ablation therapy is considered for fatal, symptomatic, and drug-resistant arrhythmias but its use is limited in pregnant women due to fetal radiation exposure.

     Radiation-free 3D mapping systems(3DMS)can visualize the origin and circuitry of arrhythmias by identifying their location through the magnetic field and radio frequency current. Ablation therapy using 3DMS can be performed without fluoroscopy. However, there are few reported cases of its use for ventricular tachycardia in pregnant women.

     We report the case of a pregnant woman with sustained ventricular tachycardia who underwent pre-pregnancy ablation; we performed 3DMS ablation in this patient at 35 weeks of gestation, considering the timing of exacerbation of sustained ventricular tachycardia, the urgency of arrhythmia, and drug resistance.

     We were able to complete the 3DMS procedure without any complications. The less invasive procedure, avoiding fetal radiation exposure, resulted in a good perinatal prognosis. Thus, 3DMS is a minimally invasive and effective treatment method for ventricular tachycardia, which can be safely used in pregnant women.

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  • Saya Yamane, Ayako Sakuma, Kumi Chogahara, Chiharu Matsubara, Akira Uc ...
    2024Volume 60Issue 2 Pages 298-303
    Published: 2024
    Released on J-STAGE: September 06, 2024
    JOURNAL RESTRICTED ACCESS

     18p tetrasomy is a chromosomal abnormality that presents with intellectual disability, eating disorders, and distinctive facial features. Peters anomaly is a group of congenital anterior ocular dysgenesis that cause opacity in the central part of the cornea. Complications such as congenital heart disease and cleft lip and palate are found in 60% of patients. We report a case of 18p tetrasomy with Peters anomaly, including a review of the literature. The patient was a 37-week-5-day-old girl. She was born with a weight of 2,212 g, a height of 44.0 cm, and a vaginal delivery. She was admitted to the hospital because of respiratory disorder and hypoglycemia. A congenital abnormalities syndrome with Peters anomaly was suspected due to left corneal opacity, distinctive facial features, a high-arched palate, internal rotation of both thumbs, and hypoplasia of the corpus callosum with MRI. Chromosome examination revealed karyotype of 47,XX,+i(18)(p10): 18p tetrasomy. 18p tetrasomy has been reported to cause strabismus as an ophthalmic finding, but no case with anterior ocular lesions has been reported, and it was considered to be an extremely rare complication.

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  • Akiko Nagasawa, Akiko Omoto, Chigusa Hirooka, Keisuke Yamamoto, Yoshik ...
    2024Volume 60Issue 2 Pages 304-309
    Published: 2024
    Released on J-STAGE: September 06, 2024
    JOURNAL FREE ACCESS

     Umbilical cord thrombosis is associated with poor perinatal outcome. We report a case of fetal posterior cloacal anomaly with ascites that resulted in fetal death due to umbilical cord thrombosis. A pregnant woman was diagnosed with fetal ascites at 25 weeks’ gestation and was referred to our hospital. Ultrasound showed fetal ascites and an irregular cyst on the dorsal side of the bladder. We suspected the cyst was hydrometra and hydrocolpos, and diagnosed the fetus had cloacal anomaly. From 30 weeks’ gestation, increased fetal ascites, enlarged cyst and oligohydramnios were gradually apparent. To prevent severe pulmonary hypoplasia, 320 ml of fetal ascites was removed at 34 weeks 0 days of gestation. Intrauterine fetal death occurred at 35 weeks 5 days of gestation and a 2,430 g girl was stillborn 2days later. Pathological autopsy confirmed posterior cloacal anomaly, hydrometra, pulmonary hypoplasia, unilateral renal atrophy and esophageal atresia(Gross C type). Placental pathology revealed an umbilical cord thrombosis, which was considered to be the main cause of fetal death. A cloacal anomaly with ascites, large hydrocolpos and oligohydramnios may have the possibility of thrombosis formation and fetal death. Therefore, careful examination in placenta and umbilical cord in such cases are important.

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  • Ayako Yanagi, Akiko Iwata, Chika Akamatsu, Shinya Kondo, Kentaro Kuras ...
    2024Volume 60Issue 2 Pages 310-316
    Published: 2024
    Released on J-STAGE: September 06, 2024
    JOURNAL FREE ACCESS

     Tetralogy of Fallot(TOF)is a congenital heart disease that is characterized by four distinct defects: over ridding of aorta, right ventricular outflow tract obstruction, ventricular septal defect, and right ventricular hypertrophy. There have been some reports of pregnancies complicated with TOF after surgical repair in Japan that resulted in deliveries; however, there have been no delivery reports on untreated TOF conditions. We present a case of maternal TOF diagnosed at the time of pregnancy in which we successfully deliver a baby at 37 weeks of gestation without surgical repair.

     The patient was a 26-year-old female, G1P0, born in another country. She was diagnosed with TOF due to the heart murmur in the first trimester of pregnancy. She presented to our hospital at 21 weeks and 2 days of gestational age, with Levine’s Classification III and NYHA Classification I. There was no cyanosis or bumble finger. The pregnancy continued under careful monitoring of the maternal cardiac function. At 37 weeks and 3 days of pregnancy, she was admitted to the hospital with tachycardia, generalized edema, marked weight gain, and elevated BNP and diagnosed with a right heart failure. The baby was delivered by cesarean section at 37 weeks and 4 days of gestational age. Endocardial repair of TOF on the mother was performed one year after the delivery.

     We experienced a case of pregnancy complicated with untreated TOF that were able to successfully bring to a full term. Careful management will continue to be necessary, as the patient’s conditions could still suddenly deteriorate, such as due to heart failure.

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  • Mao Sasaki, Takeshi Kusuda, Yoichiro Serita, Yushi Noguchi, Takashi Sh ...
    2024Volume 60Issue 2 Pages 317-322
    Published: 2024
    Released on J-STAGE: September 06, 2024
    JOURNAL FREE ACCESS

     VACTERL association consists of vertebral defects(V), anal atresia(A), cardiac malformations(C), tracheo-esophageal fistula with esophageal atresia(TE), renal defects or radial limb dysplasia(R), and limb abnormalities(L). The association is named with these initials. These multisystem morphological abnormalities are recognized at once. The incidence is estimated at approximately 1 in 10,000 to 40,000 births, and the diagnosis is based on the presence of at least three VACTERL component features. VACTERL association is associated with various other abnormalities, but central nervous system is rare. We experienced two cases of VACTERL association with Chiari malformation type 2.

     The cases were girls at 38 and 35 weeks of gestation, respectively. Both cases were diagnosed severe vertebral defects, anal atresia, abnormal renal defects, clubfoot and complicated by Chiari malformation type 2 in utero. After birth, surgery for myelomeningocele, ventriculoperitoneal shunt, and surgical intervention for anal atresia were performed.

     The morphological abnormalities of the VACTERL association are formed around the third weeks of gestation, which is similar to the time of formation of Chiari malformation type 2. We discussed the relationship between the two diseases.

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  • Chika SHIRAKURA, Yumiko ITO, Eri TSUGENO, Yumi NAKAMURA, Hajime ARAKI, ...
    2024Volume 60Issue 2 Pages 323-329
    Published: 2024
    Released on J-STAGE: September 06, 2024
    JOURNAL FREE ACCESS

     We report a case where systemic lupus erythematosus(SLE)was diagnosed in a patient with pleurisy during pregnancy. The patient was a 29-year-old primiparous woman who had been treated for rheumatoid arthritis(RA)at the orthopedic department in our hospital and was prescribed Salazosulfapyridine(SASP), which improved symptoms of RA. She conceived spontaneously, at which point SASP was discontinued, then visited our department at 19 weeks’ gestation for consultation due to the risk factor with RA. As the patient did not report any subjective symptoms at this point, it was agreed to have prenatal checkup at her primary facility. At 26 weeks’ gestation, she experienced difficulty breathing and visited the orthopedics department, but the symptoms did not improve. At 27 weeks’ gestation, she was referred to our department. At that point she was experiencing tachycardia, tachypnea, and orthopnea, and was hospitalized. Because of the high likelihood of premature birth, she was given Betamethasone in order to promote fetal lung maturation, which subsequently improved tachypnea, and orthopnea was no longer observed. SLE diagnosis was later confirmed, and we attributed her symptoms to pleurisy and pericarditis. Her symptoms improved with a steroid treatment, and we determined that the pregnancy was maintainable. She was discharged at 30 weeks’ gestation and had a live birth vaginally at 37 weeks’ gestation. SLE is a common disease in women of reproductive age, and its onset and flares during pregnancy have often been reported. This case showed that pregnancy could be maintained with immunosuppressive therapy, even when early termination of pregnancy is considered due to severe symptoms of autoimmune diseases.

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  • Kensuke Fujishiro, Yuichi Kubo, Hiroki Otsuka, Masaya Kibe, Hiroshi Oh ...
    2024Volume 60Issue 2 Pages 330-334
    Published: 2024
    Released on J-STAGE: September 06, 2024
    JOURNAL FREE ACCESS

     Congenital glycosylphosphatidylinositol(GPI)deficiency is caused by mutations in genes responsible for synthesizing GPI anchors that bind various proteins to the cell membrane, resulting in intellectual disability, motor retardation, and epilepsy. This rare disease affects only about 50 patients in Japan and poses diagnostic challenges, particularly in cases without a family history of GPI. The primary symptoms include intellectual disability and motor developmental disorder without perinatal abnormalities. Herein, we report on a patient with congenital GPI deficiency who was transferred to the neonatal intensive care unit due to severe asphyxia.

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